Clinical Characteristics And Outcomes Of Abdominal Desmoplastic Small Round Cell Tumor: A Single-Center Experience Of 10 Cases

Background: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive abdominal tumor typically arising in adolescent and young adult. With no large series reported, the optimal therapy modalities remain unclear. Methods: Clinical data of all consecutive DSRCT patients treated in Cancer Hospital Chinese Academy of Medical Sciences between January 2000 and December 2014 were reviewed. Results: Ten patients with a median age of 27 years (range 14-52 years) were identified; 9 male and 1 female. 9 patients underwent surgery, and the other 1 patient received biopsy. Among the 9 patients who underwent surgery, 5 patients (55.6%) underwent complete surgical removal of all macroscopic peritoneal disease and 4 patients (44.4%) underwent palliative resection. 1 patient received preoperative chemotherapy, and all the 9 patients who underwent surgery received postoperative adjuvant chemotherapy. 2 patients had intensity modulated whole abdominopelvic radiotherapy (WAP RT), and 1 patient received TARCEVA. After a median follow-up of 30 months, the median OS was 26 months. The median OS of the patients with complete surgical removal was better than the patients with no complete surgical removal (33 months: 14 months; Log Rank test, P<0.05). Conclusions: A multimodal treatment combining systemic chemotherapy, complete macroscopic resection, and postoperative whole abdominopelvic-intensity modulated radiotherapy (WAP-IMRT) could improve survival.